Significance of histopathologic examination in the diagnosis of dentin defects associated with type IV osteogenesis imperfecta: two case reports.

Two cases of type IV osteogenesis imperfecta, which is divided into subtypes A and B on the basis of the absence or the presence of dental alterations, respectively, are examined with respect to their dental features clinically, radiographically, and histopathologically. There were no characteristic dental abnormalities noted on the clinical and radiographic examination. However, the histopathologic examination with both light and electron microscopy disclosed characteristic dentin defects such as unevenly calcified matrixes, irregular tubular patterns, obliterated dentinal tubules, and cellular inclusions in the circumpulpal dentin of primary teeth. As a result, the patients were diagnosed as having osteogenesis imperfecta type IVB, although the clinical dental alterations were scarcely apparent. These findings indicate the importance of histopathologic examination with light and transmission electron microscopy for accurate diagnosis of osteogenesis imperfecta.